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Cancer Health Center

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Genetics of Colorectal Cancer (PDQ®): Genetics - Health Professional Information [NCI] - Major Genetic Syndromes

Table 9. Clinical Practice Guidelines for Colon Surveillance of Attenuated Familial Adenomatous Polyposis (AFAP) continued...

Adenomas, serrated adenomas, and hyperplastic polyps can be seen in MAP patients. The CRCs tend to be right-sided and synchronous at presentation and seem to carry a better prognosis than sporadic CRC.[192] Clinical management guidelines for biallelic MAP range between once a year to every 3 years for colonoscopic surveillance beginning at age 18 to 30 years,[92,185,188] with upper endoscopic surveillance beginning at age 25 to 30 years.[185] (Refer to Table 10 for more information about available clinical practice guidelines for colon surveillance in biallelic MAP patients.) The recommended upper endoscopic surveillance interval can be based on the burden of involvement according to Spigelman criteria.[185] Total colectomy with ileorectal anastomosis or subtotal colectomy may be appropriate for patients with MYH-associated polyposis, provided that they have no rectal cancer or severe rectal polyposis at presentation and that they undergo yearly endoscopic surveillance thereafter.[188,193]

Table 10 summarizes the clinical practice guidelines from different professional societies regarding colon surveillance of biallelic MAP.

Table 10. Clinical Practice Guidelines for Colon Surveillance of BiallelicMYH-Associated Polyposis (MAP)

OrganizationConditionScreening MethodScreening FrequencyAge Screening InitiatedComment
IPAA = ileal pouch-anal anastomosis; IRA = ileorectal anastomosis; NCCN = National Comprehensive Cancer Network.
a Fewer than 20 adenomas that are each <1 cm in diameter and without advanced histology so that colonoscopy with polypectomy can be used to effectively eliminate the polyps.
Europe Mallorca Group (2008)[185]BiallelicMYHmutation carrierColonoscopyEvery 2 y18-20 y
Nieuwenhuis et al. (2012)[188]BiallelicMYHmutation carrierColonoscopyEvery 1-2 y
NCCN (2014)[92]Personal history of MAP, small adenoma burdenaColonoscopyEvery 1-2 yIf patient had colectomy with IRA due to significant polyposis not manageable with polypectomy, endoscopic evaluation every 6-12 mo depending on polyp burden.
Colectomy and IRA may be considered in patients aged ≥21 y
NCCN (2014)[92]Personal history of MAP with significant polyposisNot applicableNot applicableNot applicableColectomy with IRA preferred. Consider proctocolectomy with IPAA if dense rectal polyposis. If patient had colectomy with IRA, then endoscopic evaluation of rectum every 6-12 mo depending on polyp burden.
NCCN (2014)[92]Unaffected, at-risk family member; family mutation unknown;MYHmutation status unknown or positive (biallelic)ColonoscopyEvery 2-3 y25-30 yIf positive for a singleMYHmutation, follow average-risk colorectal screening.
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